EXTL3 Antibody

Code CSB-PA03075A0Rb
Size US$166
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  • Western blot
    All lanes: EXTL3 antibody at 2µg/ml
    Lane 1: EC109 whole cell lysate
    Lane 2: 293T whole cell lysate
    Secondary
    Goat polyclonal to rabbit IgG at 1/15000 dilution
    Predicted bane side: 105 kDa
    Observed band side: 105 kDa

  • Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA03075A0Rb at dilution of 1:100

  • Immunofluorescent analysis of Hela cells using CSB-PA03075A0Rb at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) EXTL3 Polyclonal antibody
Uniprot No.
Target Names
EXTL3
Alternative Names
botv antibody; DKFZp686C2342 antibody; Exostoses (multiple)-like 3 antibody; Exostoses-like 3 antibody; Exostosin-like 3 antibody; EXT-related protein 1 antibody; EXTL1L antibody; EXTL3 antibody; EXTL3_HUMAN antibody; EXTR1 antibody; Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferas antibody; Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase antibody; Hereditary multiple exostoses gene isolog antibody; KIAA0519 antibody; Multiple exostosis-like protein 3 antibody; Putative tumor suppressor protein EXTL3 antibody; REG antibody; Reg receptor antibody; REGR antibody; RPR antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Exostosin-like 3 protein (2-245AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The EXTL3 Antibody (Product code: CSB-PA03075A0Rb) is Non-conjugated. For EXTL3 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA03075B0Rb EXTL3 Antibody, HRP conjugated ELISA
FITC CSB-PA03075C0Rb EXTL3 Antibody, FITC conjugated
Biotin CSB-PA03075D0Rb EXTL3 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:20-1:200
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Glycosyltransferase which regulates the biosynthesis of heparan sulfate (HS). Important for both skeletal development and hematopoiesis, through the formation of HS proteoglycans (HSPGs). Required for the function of REG3A in regulating keratinocyte proliferation and differentiation.
Gene References into Functions
  1. Human lumenal N-glycosylated EXTL3 (EXTL3DeltaN) was cloned, expressed in human embryonic kidney cells, and purified. Various biophysical and biochemical approaches were then employed to elucidate the N-glycosylation sites and the function of their attached N-glycans. PMID: 29346724
  2. EXTL3 missense mutation is associated with spondylo-epi-metaphyseal dysplasia. PMID: 28331220
  3. EXTL3 mutations as a novel cause of severe immune deficiency with skeletal dysplasia and developmental delay and underline a crucial role of HS in thymopoiesis and skeletal and brain development. PMID: 28148688
  4. We show that biallelic mutations in EXTL3 disturb glycosaminoglycan synthesis and thus lead to a recognizable syndrome characterized by variable expression of skeletal, neurological, and immunological abnormalities. PMID: 28132690
  5. Recombinant EXTL2 showed weak ability to transfer N-acetylgalactosamine to heparan sulfate precursor molecules but also, that EXTL2 exhibited much stronger in vitro N-acetylglucosamine-transferase activity related to elongation of heparan sulfate chains. PMID: 25829497
  6. Regenerating islet-derived 1alpha (Reg-1alpha) protein is new neuronal secreted factor that stimulates neurite outgrowth via exostosin Tumor-like 3 (EXTL3) receptor. PMID: 22158612
  7. Lysosomal glycosaminoglycan levels in mucopolysaccharidosis are reduced by EXTL3 gene silencing. PMID: 19690583
  8. We conclude that EXTL3 promoter methylation and its related loss of EXTL3 expression are involved in the loss of HS expression in mucinous CRCs. PMID: 18543267
  9. HIP enhanced EXTL3 translocation from the membrane to the nucleus, in support of a model whereby EXTL3 mediates HIP signaling for islet neogenesis. PMID: 19158046
  10. A missense mutation involving the exon 3 of the EXTL3 gene in the case of obstructing colon cancer is described in a 31-year-old patient affected by hereditary multiple exostoses. PMID: 19653241
  11. These results suggest that EXTL3/EXTR1 is a cell surface Reg receptor that binds to Reg protein. PMID: 10753861

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Involvement in disease
Immunoskeletal dysplasia with neurodevelopmental abnormalities (ISDNA)
Subcellular Location
Endoplasmic reticulum membrane; Single-pass type II membrane protein. Golgi apparatus.
Protein Families
Glycosyltransferase 47 family
Tissue Specificity
Ubiquitous. Expressed in keratinocytes.
Database Links

HGNC: 3518

OMIM: 605744

KEGG: hsa:2137

STRING: 9606.ENSP00000220562

UniGene: Hs.491354

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